TY  - CHAP
M1  - Book, Section
TI  - Small Bowel Cancer and Appendiceal Tumors
A1  - Gulhati, Pat
A1  - Shen, John Paul
A1  - Raghav, Kanwal P.
A1  - Overman, Michael J.
A2  - Kantarjian, Hagop M.
A2  - Wolff, Robert A.
A2  - Rieber, Alyssa G.
Y1  - 2022
N1  - 
T2  - The MD Anderson Manual of Medical Oncology, 4e
AB  - KEY  CONCEPTSSmall  bowel  neoplasms  are  rare  with  roughly  12,000  new  cases  per  year  in  the  United  States.  The  two  most  common  histologies  are  adenocarcinoma  (30%–40%)  and  carcinoid  tumors  (40%–45%).Inflammatory  bowel  disease,  Peutz-Jeghers,  familial  adenomatous  polyposis,  celiac  disease,  and  hereditary  nonpolyposis  colorectal  cancer  and  all  increase  the  risk  of  developing  small  bowel  adenocarcinomas.Surgery  is  the  mainstay  of  curative  therapy;  however,  patients  often  present  with  more  advanced  disease,  especially  with  adenocarcinomas.There  is  no  firmly  established  adjuvant  therapy  for  patients  with  either  carcinoid  tumors  or  adenocarcinoma.Systemic  therapy  for  patients  with  advanced  disease  mirrors  treatment  for  neuroendocrine  tumors  for  carcinoids,  whereas  FOLFOX  (modified  5-fluorouracil  and  oxaliplatin),  CAPOX,  and  regimens  with  irinotecan  are  active  for  patients  with  adenocarcinoma.The  molecular  defects  in  adenocarcinomas  are  being  elucidated.  To  date,  however,  no  targeted  therapy  has  an  established  role  in  advanced  disease.  Mismatch  repair  defects  (even  more  frequent  than  in  colorectal  cancer)  are  predictive  of  response  to  immune  checkpoint  blockade.Appendiceal  neoplasms  are  very  rare  consist  of  two  major  types:  appendiceal  carcinoid  tumors  and  appendiceal  epithelial  tumors,  each  accounting  for  roughly  half  all  cases.No  clear  risk  factors  for  appendiceal  neoplasms  have  been  identified.Surgery  is  the  mainstay  of  curative  therapy  for  all  patients  with  appendiceal  neoplasms.  Patients  with  adenocarcinomas  are  more  likely  to  present  with  advanced  disease  in  comparison  with  those  with  carcinoid  tumors.The  histologic  subtypes  of  epithelial  appendiceal  neoplasms  include  low-  and  high-grade  appendiceal  mucinous  neoplasms  and  invasive  adenocarcinomas  that  may  be  mucinous  (low  or  high  grade),  nonmucinous,  or  with  signet  ring  cell  features.Metastatic  low-grade  mucinous  tumors  have  a  more  indolent  disease  course,  respond  poorly  to  systemic  therapy,  and  are  primarily  managed  with  surgical  cytoreduction  and  hyperthermic  intraperitoneal  chemotherapy  (HIPEC).Metastatic  high-grade  adenocarcinomas,  which  include  all  tumors  with  signet  ring  cell  features,  are  often  treated  with  systemic  chemotherapy.  The  benefit  from  cytoreductive  surgery  and  HIPEC  is  uncertain.  
SN  - 
PB  - McGraw Hill Education
CY  - New York, NY
Y2  - 2024/04/23
UR  - hemonc.mhmedical.com/content.aspx?aid=1190835670
ER  -